Rev Esp Med Nucl Imagen Mol. 2015;34(4):270–271
Interesting image
Urachal bladder in kidney transplanted patient with Prune Belly syndrome and patent urachus Vejiga uracal postrasplante renal en paciente con síndrome de Prune Belly y uraco persistente M. Sánchez Aguilar a,∗ , F.J. García Gómez a , J.I. Cuenca Cuenca a , J.L. Tirado Hospital a , F. de la Cerda Ojeda b , A. Sánchez Moreno b a b
Nuclear Medicine Department, Virgen del Rocío Universitary Hospital, Seville, Spain Pediatric Nephrology Department, Virgen del Rocío Universitary Hospital, Seville, Spain
We report a 16-year-old-male patient with Prune-Bellysyndrome (PBS) and patent urachus, bilateral renal dysplasia and end-stage renal disease (ESRD). The first kidney transplantation was performed at the age of 3 years, leaving an urachal catheter. Transplant failure underwent in a new cadaveric transplant at the present. Post-kidney transplant renogram was performed to evaluate the renal function after the administration of 150 MBq of 99m Tcmercapto-acetyl-triglycine and revealed adequate perfusion with a homogeneous radiotracer uptake in the renal transplant (Fig. 1A). Elimination phase (Fig. 1B) showed a progressively increasing
tracer accumulation in “urachal bladder” (red-arrow), without activity in the urinary bladder (white-arrow). PBS is a congenital abnormality of unknown aetiology with characteristic features: deficient development of abdominal muscles that causes the skin of the abdomen to wrinkle like a prune, cryptorchidism, abnormalities of the urinary tract, chronic renal failure and ESRD.1 The incidence is estimated between 1/35,000 and 1/50,000 live births while 97% of patients are male.2 Urethral hypoplasia or atresia is present in around 18% of cases, leading to a very poor prognosis and death unless there was an associated patent urachus or vesico-cutaneous fistula.3 The reported
A
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B
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Fig. 1. 99m Tc-mercapto-acetyl-triglycine post-kidney transplant renogram. (A) Vascular phase, showing good perfusion without morphological changes. (B) Elimination phase, revealing a patent urachus with a functional “urachal bladder” (red arrow), without significant activity being observed in the urinary bladder (white arrow).
∗ Corresponding author. E-mail address: [email protected] (M. Sánchez Aguilar). http://dx.doi.org/10.1016/j.remn.2015.01.001 2253-654X/© 2014 Elsevier España, S.L.U. and SEMNIM. All rights reserved.
M. Sánchez Aguilar et al. / Rev Esp Med Nucl Imagen Mol. 2015;34(4):270–271
average age at transplantation usually does not exceed 15-yearsold. Awareness of PBS in renogram would contribute to the correct diagnosis. Conflict of interest The authors have no funding or conflicts of interest to declare.
271
References 1. Jennings RW. Prune belly syndrome. Semin Pediatr Surg. 2000;9:115–20. 2. Herman TE, Siegel MJ. Prune belly syndrome. J Perinatol. 2009;29:69–71. 3. Sarhan OM, Al-Ghanbar MS, Nakshabandi ZM. Prune belly syndrome with urethral hypoplasia and vesico-cutaneous fistula: a case report and review of literature. Urol Ann. 2013;5:296–8.
Interesting image
Urachal bladder in kidney transplanted patient with Prune Belly syndrome and patent urachus Vejiga uracal postrasplante renal en paciente con síndrome de Prune Belly y uraco persistente M. Sánchez Aguilar a,∗ , F.J. García Gómez a , J.I. Cuenca Cuenca a , J.L. Tirado Hospital a , F. de la Cerda Ojeda b , A. Sánchez Moreno b a b
Nuclear Medicine Department, Virgen del Rocío Universitary Hospital, Seville, Spain Pediatric Nephrology Department, Virgen del Rocío Universitary Hospital, Seville, Spain
We report a 16-year-old-male patient with Prune-Bellysyndrome (PBS) and patent urachus, bilateral renal dysplasia and end-stage renal disease (ESRD). The first kidney transplantation was performed at the age of 3 years, leaving an urachal catheter. Transplant failure underwent in a new cadaveric transplant at the present. Post-kidney transplant renogram was performed to evaluate the renal function after the administration of 150 MBq of 99m Tcmercapto-acetyl-triglycine and revealed adequate perfusion with a homogeneous radiotracer uptake in the renal transplant (Fig. 1A). Elimination phase (Fig. 1B) showed a progressively increasing
tracer accumulation in “urachal bladder” (red-arrow), without activity in the urinary bladder (white-arrow). PBS is a congenital abnormality of unknown aetiology with characteristic features: deficient development of abdominal muscles that causes the skin of the abdomen to wrinkle like a prune, cryptorchidism, abnormalities of the urinary tract, chronic renal failure and ESRD.1 The incidence is estimated between 1/35,000 and 1/50,000 live births while 97% of patients are male.2 Urethral hypoplasia or atresia is present in around 18% of cases, leading to a very poor prognosis and death unless there was an associated patent urachus or vesico-cutaneous fistula.3 The reported
A
Image:1-3
Image:4-6
Image:7-9
Image:10-12
Image:13-15
Image:16-18
Image:19-21
Image:22-24 Image:25-27
Image:28-30
B
Image:2-5
Image:26-29
Image:6-9
Image:10-13
Image:14-17
Image:18-21
Image:22-25
Image:30-33
Image:34-37
Image:38-41
Image:42-45
Image:46-49
Fig. 1. 99m Tc-mercapto-acetyl-triglycine post-kidney transplant renogram. (A) Vascular phase, showing good perfusion without morphological changes. (B) Elimination phase, revealing a patent urachus with a functional “urachal bladder” (red arrow), without significant activity being observed in the urinary bladder (white arrow).
∗ Corresponding author. E-mail address: [email protected] (M. Sánchez Aguilar). http://dx.doi.org/10.1016/j.remn.2015.01.001 2253-654X/© 2014 Elsevier España, S.L.U. and SEMNIM. All rights reserved.
M. Sánchez Aguilar et al. / Rev Esp Med Nucl Imagen Mol. 2015;34(4):270–271
average age at transplantation usually does not exceed 15-yearsold. Awareness of PBS in renogram would contribute to the correct diagnosis. Conflict of interest The authors have no funding or conflicts of interest to declare.
271
References 1. Jennings RW. Prune belly syndrome. Semin Pediatr Surg. 2000;9:115–20. 2. Herman TE, Siegel MJ. Prune belly syndrome. J Perinatol. 2009;29:69–71. 3. Sarhan OM, Al-Ghanbar MS, Nakshabandi ZM. Prune belly syndrome with urethral hypoplasia and vesico-cutaneous fistula: a case report and review of literature. Urol Ann. 2013;5:296–8.
